Evaluation and treatment of inherited pheochromocytoma in pregnancy

Abstract

Pheochromocytomas represent a rare, but potentially devastating, cause of hypertension among pregnant patients. Inherited predisposition to these tumors requires vigilant screening, especially in women of child-bearing age. We present an illustrative case of a multiple endocrine neoplasia type 2A patient who was successfully surgically treated for a pheochromocytoma during pregnancy. A review of the existing, English-language literature and screening guidelines was undertaken. Pheochromocytomas presenting during pregnancy, can be safely and effectively treated with laparoscopic surgery, allowing the maintenance of a normal pregnancy. Screening of patients with inherited endocrinopathies that predispose to adrenal medullary tumors must be adapted to maximize the opportunity to detect a pheochromocytoma in the pregravid state. More frequent testing and a lower threshold for biochemical or radiologic investigation are suggested for women in their reproductive years.